Ichthyosis linearis circumflexa is a distinctive skin condition of generalized hyperkeratosis and polycyclic and serpiginous erythematous plaques with a. It manifests as collodion baby at birth and refractory erythroderma during infancy and early childhood days. Ichthyosis linearis circumflexa (ILC). A 7 years old girl, born of a non-consanguineous marriage, presented with multiple asymptomatic dry and scaly patches over her body since infancy. The.

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Get free access to newly published articles. Elsevier Saunders Company; Introduction Ichthyosis linearis circumflexa is a rare, autosomal recessive disorder of keratinization characterized by annular and polycyclic lesions with double-edged scales. Purchase access Subscribe to the journal.

Ichthyosis linearis circumflexa – Wikipedia

References Richard G, Ringpfeil F. A diagnosis of ichthyosis linearis circumflexa was established based on the clinical and histopathological findings. Privacy Policy Terms of Use.

Ichthyosis linearis circumflexa is a rare form of ichthyosis characterized by polycyclic and annular lesions which are bordered by a double-edged scale. Author information Article notes Copyright and License information Disclaimer.

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National Center for Biotechnology InformationU. Serum biochemistries, IgE levels and complete blood count were within reference intervals.


Ichthyosis Linearis Circumflexa as the Only Clinical Manifestation of Netherton Syndrome.

Sign in to access your subscriptions Sign in to your personal account. If you have any concerns with your skin or its treatment, see a dermatologist for advice. The patient had not benefited from ciecumflexa and topical corticosteroids.

In some cases there is no family history of the trait and Netherton linsaris is revealed when two unaffected parents who are both carriers of the mutated recessive gene have a child who receives both copies of the recessive gene.

J Eur Acad Dermatol Venerol. Sign in to make a comment Sign in to your personal account. Netherton syndrome should be at the top of the differential diagnosis list in a newborn with erythroderma and abnormal-looking scalp hairor in an older child with ichthyosis linearis circumflexa and sparse lustreless hair.

On dermatological examination, widespread erythematous, annular, polycyclic and double-edged papulosquamous plaques were detected on the trunk and extremities [ Fig. Netherton syndrome is inherited as an autosomal recessive trait.

Netherton syndrome

A,B Clinical improvement of skin lesions after phototherapy. Other signs a patient may develop sometime throughout their lifetime include:. Ichthyossis for email alerts with links to free full-text articles Access PDFs of free articles Manage your interests Save searches and receive search alerts.

The exact mechanism of action of phototherapy in this condition is unknown, but it has been citcumflexa that phototherapy may enhance the synthesis of other serine protease inhibitors that compensate the activity of the defective LEKTI function. Create a free personal account to download free article PDFs, sign up for alerts, customize your interests, and more.

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There is no specific treatment for Netherton syndrome. On the face, there was a mild, diffuse erythema with slight scaling. A year-old patient diagnosed as ichthyosis linearis circumflexa demonstrated considerable improvement with narrowband UVB phototherapy at the end of 30 sessions.

Narrowband UVB phototherapy as a novel treatment for Netherton syndrome. Self-skin examination New cirdumflexa apps to check your skin Learn more Sponsored content. The treatment was well tolerated and the patient remained in remission for five months after discontinuation of phototherapy.

Altman J, Stroud J.

Periods of little or no disease symptoms are interspersed with intermittent exacerbations. LEKTI is a new type of serine protease inhibitor with antitrypsin activity. The majority of lesions cleared after 30 sessions with a cumulative dose of